Terreros D A, Knight J A, Peric-Golia L, Cheung A K
Laboratory Service, Veterans Administration Medical Center, Salt Lake City, UT 84148.
Arch Pathol Lab Med. 1989 Jan;113(1):31-5.
Generalized beta 2-microglobulin deposition in the tissues of a 64-year-old man with idiopathic end-stage renal disease was determined post mortem by the use of immunoperoxidase and radial immunodiffusion techniques. He developed progressive organ failure secondary to beta 2-microglobulin deposition, requiring dialysis for the last two years of his life. His immediate cause of death was severe bronchopneumonia. The extensive tissular deposits were shown to be congophilic but not birefringent by alkaline Congo red stains. Immunochemical studies showed that the deposits were beta 2-microglobulin-positive but AA amyloid-, P-component-, prealbumin-, and kappa and lambda light chain-negative. We suggest that this patient had a primary beta 2-microglobulin dyscrasia in which beta 2-microglobulin deposited in tissues but did not undergo complete amyloidogenic change.
一名64岁特发性终末期肾病男性患者，其组织中存在广泛性β2微球蛋白沉积，在尸检时通过免疫过氧化物酶和放射免疫扩散技术得以确定。他因β2微球蛋白沉积继发进行性器官衰竭，在生命的最后两年需要透析治疗。其直接死因是严重支气管肺炎。碱性刚果红染色显示，广泛的组织沉积物呈嗜刚果红性，但无双折射现象。免疫化学研究表明，沉积物β2微球蛋白呈阳性，但AA淀粉样蛋白、P成分、前白蛋白以及κ和λ轻链均呈阴性。我们认为，该患者存在原发性β2微球蛋白异常血症，其中β2微球蛋白沉积于组织中，但未发生完全的淀粉样变。
